Sclerotherapy of esophageal varices in hemophilia patients with liver cirrhosis - a prospective, controlled clinical study.

INTRODUCTION: Bleeding from esophageal varices is a serious clinical condition in hemophilia patients due to congenital deficiency or lack of clotting factors VIII (in hemophilia A) and IX (in hemophilia B), decreased clotting factor II, VII, IX, X synthesis in the course of chronic liver disease and hipersplenic thrombocytopenia. The aim of this study was to assess the efficacy and safety of endoscopic sclerotherapy in acute esophageal variceal bleeding and in secondary prophylaxis of hemorrhage. The aim was also to investigate the optimal activity of deficiency factors VIII or IX and duration of replacement therapy required to ensure proper hemostasis after sclerotherapy procedures. MATERIAL AND METHODS: 22 hemophilia patients (A-19, B-4) with coexistent liver cirrhosis and active esophageal variceal bleeding treated with endoscopic sclerotherapy were subjected to prospective analysis. The patients who survived were qualified to repeated sclerotherapy procedures every 3 weeks within secondary prophylaxis of bleeding (investigated group). A 3-day substitution therapy enhanced the infusion of the deficient or lacking factor in doses allowing to reach 80-100% of normal value activity of factor VIII on the 1st day and 60-80% in the next two days. The desired activity of factor IX was 60- 80% and 40-60% respectively. The control group consisted of 20 non-hemophiliac patients with liver cirrhosis comparable in terms of age, sex, stage of advancement of liver cirrhosis, who underwent the same medical proceedings as the investigated group. RESULTS: Active esophageal bleeding was stopped in 21 of 22 (95%) hemophilia patients. Complications were observed in 3 patients; 2 patients died. The rate of hemostasis, complications and deaths in the control group were comparable and no statistical differences were found. In hemophilia patients subjected to secondary prophylaxis of hemorrhage, in 18 of 20 (80%), complete eradication of esophageal varices was achieved after 4 to 7 sclerotherapy procedures in 1 patient (average 5.4). Recurrent bleeding was observed in 15% of patients, complication in 20%; 1 patient died. Time lapse from bleeding to eradication was 12-21 weeks (average 15.2). In the control group the rate of variceal eradication, complication and deaths was comparable and no statistical differences were found. The usage of factor VIII concentrates was as follows: in hemophilia A, in a severe form - 80.9 U/kg b.w./day, in hemophilia A in a severe form with an inhibitor <5 BU - 95.2 U/kg b.w./day, in mild form - 64.2 U/kg b.w./day and in severe hemophilia B - 91.6 U/kg b.w./day. CONCLUSIONS: Sclerotherapy is an effective method in the management of esophageal variceal bleeding in hemophilia patients. It is also effective for total eradication of varices when applied as a secondary prophylaxis of hemorrhage. In our opinion, a 3-day replacement therapy at the applied doses is sufficient to ensure hemostasis and avoid bleeding complications.

Sclerotherapy Of Esophageal Varices In Severe Hemophilia A Patient And High Titer Inhibitor--Case Report.

In cirrhotic hemophilia patients bleeding from esophageal varices is a serious clinical condition due to congenital deficiency of clotting factors VIII or IX, decreased prothrombin synthesis and hypersplenic thrombocytopenia. In hemophiliac with high-titer inhibitor bypassing therapy is required with activated prothrombin complex concentrates (aPCC) or recombinant activated coagulation factor VII (rFVIIa). Doses and duration treatment with these agents following endoscopic treatment of esophageal varices have not been yet established. Authors report the first case of a severe hemophilia A patient with high titer inhibitor (40 BU) treated with repeated injection sclerotherapy. The patient was admitted with symptoms of massive esophageal variceal hemorrhage ceased with emergency sclerotherapy. Bypassing therapy was administered with aPCC at initial dose of 72.5 U/kg and then with average daily dose of 162 U/kg through 5 days. To achieved a total eradication of esophageal varices the patient was then subjected to four elective sclerotherapy procedures. Two were covered by aPCC with daily dose of 120 U/kg and 145 U/kg for 4 and 3 days respectively and the following two procedures were covered by rFVIIa with the initial dose of 116 µg/kg and the next doses of 87 µg/kg administered every 3 hours in procedure day and every 4 hours on the next two days. During all procedures excellent hemostasis was achieved and no hemorrhagic or thromboembolic complications were observed. Bypassing regimen therapy with aPCC and rFVIIa we applied have been shown to be safe and effective in this patient subjected to sclerotherapy procedures.

Radiofrequency ablation in the management of Barrett's esophagus - preliminary own experience.

INTRODUCTION: Barrett's esophagus develops as a result of chronic injury of esophagus epithelium from gastroesophageal reflux disease. It is defined when metaplastic columnar epithelium replaces the stratified squamous epithelium which normally lies in the distal esophagus. The condition represents a risk factor for esophageal adenocarcinoma. The aim of the radiofrequency ablation (RFA) method is to destroy metaplastic epithelium with radiofrequency electric current and to stimulate reappearance of the flat multilayer epithelium in the distal esophagus. AIM: To evaluate the efficiency and safety of the RFA technique, newly introduced in Poland, in the management of Barrett's esophagus. MATERIAL AND METHODS: Twelve patients were treated with the RFA method. Patients with Barrett's esophagus confirmed in the histopathological report were qualified for treatment. Two RFA techniques were applied using a BARRX(®) device: circular based on the balloon HALO(360) system or focal based on the HALO(90) system mounted to the endoscopic ending. The procedures were performed at 2-month intervals. The macroscopic and microscopic effects of RFA therapy, the patients' treatment tolerance as well as potential complications were evaluated. RESULTS: In the group of 12 patients subjected to RFA therapy, 10 completed the therapeutic cycle. A total of 37 procedures were performed: 5 HALO(360) and 32 HALO(90). In all patients eradication of the abnormal metaplastic esophageal epithelium was achieved, as confirmed in both endoscopic and histopathological evaluation. In 2 patients with ongoing therapy progressive eradication of metaplastic epithelium was observed. No significant RFA-related complications were reported. CONCLUSIONS: Based on our preliminary results we consider this method to be promising, free of significant complications and well tolerated by patients. In most patients it results in successful eradication of metaplastic epithelium in the distal esophagus.